Pediatric Dental Health

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Sickle Cell Disease
Sickle cell disease is an inherited, chronic, blood
disease in which the red blood cells become crescent-shaped and function
abnormally. The sickle-shaped blood cells block the smallest vessels in the
body's organs, thereby reducing their supply of oxygen, and causing severe
damage to the organs. Sickle cell disease is caused by an abnormal type of
hemoglobin - hemoglobin S.

Sickle cell disease affects one out of every 600 African-Americans, and occurs
in 0.15% of African-American newborns. Eight percent of black Americans have
sickle cell trait, which does not usually cause medical problems. In the United
States, patients with sickle cell disease survive into their fifth and sixth
decades of life.

Research on sickle cell disease was initiated at the end of World War II. In
1945, Dr. William B. Castle discovered that the sickled red blood cells of
patients affected by the disease were only found in the veins, that is, in the
deoxygenated blood. This meant that the sickling occurred after the red blood
cells had released the life-giving oxygen. This gave future researchers a clue
as to where the ultimate cause of sickle cell disease might be found. In 1949
Dr. Linus Pauling, America's leading physical chemist of the time, published a
landmark scientific paper entitled "Sickle Cell Anemia, a Molecular
Disease." In his famous paper, he and his research team demonstrated that
the hemoglobin of sickle cell anemia patients had a different electrical charge
from that of healthy people. This meant that the hemoglobin molecule was somehow
different in these sick patients. Hemoglobin is the molecule in red blood cells
which binds to oxygen, and then transports the oxygen to distant parts of the
body. Dr. Pauling's discovery was very significant, because it showed that a
very small change in the molecular structure of a protein could lead to the
development of a devastating disease. In 1957 Dr. Vernon Ingram used a new
technique (paper chromatography combined with electrophoresis) for separating
different proteins. Using his new protein separation technique, he determined
that there was only one amino acid which was different in the hemoglobin
molecule of sickle cell patients. He found that the amino acid, valine, was
substituted in place of the usual amino acid, glutamic acid, in the beta chain
of the hemoglobin molecule. His new discovery would later be shown to be an
example of single nucleotide polymorphism.

Sickle cell disease is chronic illness, punctuated by multiple crises, which can
become life-threatening at any time. A sickling crisis in children can be caused
by lack of oxygen in any part of the body, dehydration, infection - which can
lead to acidosis, fatigue, or stress. Death in children who have sickle cell
disease can be caused by infection, acute chest syndrome, splenic sequestration,
or other causes.

Signs Of Sickle Cell Disease In Children:

The vaso-occlusive crisis is the most frequent sign in children. In
infants, it appears as a painful swelling of both hands and feet (hand-foot
syndrome), and is often the first sign of sickle cell anemia in children.

Later on in life, these painful vaso-occlusive crises occur in the hip,
knee, chest, and back joints.

The acute chest syndrome. This is an acute respiratory tract illness,
which is associated with fever, chest pain, and respiratory distress. It
tends to affect children who are older than three years of age.

Episodes of abdominal pain, often excruciating.

Sequestration crisis. This is a painful increase in the size of the
spleen, due to a blockage of the blood vessels in the spleen. This leads to
acute hyperspleenism.

What The Dentist Needs To Do:

Consult with the child's primary pediatrician.

Request the child's most recent lab test results.

Schedule the dental appointment in the morning, when the child is
well-rested.

Prescribe additional antibiotics, before and after invasive dental
treatment, to prevent infections.

Recommend adequate fluid intake before the dental appointment.

Consider using nitrous oxide to reduce anxiety during dental treatment.

Initiate a strict preventive dental program for the child. This will help
prevent dental disease and infections.

A recent study published in The New England Journal of Medicine
found that the acute chest syndrome was the leading cause of death in
patients with sickle cell disease. The most common causes of death for the
538 patients with sickle cell disease in the study were pulmonary fat emboli
and bronchopneumonia.Vichinsky EP, et al.: Causes and outcomes of the acute chest syndrome
in sickle cell disease. The New England Journal of Medicine. 22 June 2000;
342(25) 1855-1865.

Does Your Child Need A
Mouthguard?
An estimated 25 million youths in the United States
participate in competitive school sports, and 20 million children and
adolescents play organized out-of-school sports. Sports-related dental trauma
continues to occur, but there is something we can do about it.

Properly designed and custom fabricated mouthguards are essential in
preventing athletic oral-facial injury. The use of mouthguards reduces the
likelihood of tooth fractures and tooth dislocations. Mouthguards protect
against jaw fractures by absorbing the energy of a traumatic blow to the chin.
They also protect against soft tissue lacerations, and against bruising of the
lips and cheeks. Finally, mouthguards reduce the likelihood of concussions,
cerebral hemorrhage, and unconsciousness during athletic activities.

In the early 1930's boxers began using rubber mouthguards to protect their
teeth and lips during fights. Later, in the 1950's, the American Dental
Association began promoting the use of mouthguards among athletes. In 1973 the
National Collegiate Athletic Association required players to wear mouthguards
during games. In 1995 the American Dental Association amended its
recommendations for oral-facial protection to include all sports participants
who were at risk for injury. Currently all states mandate the use of
mouthguards during high school football, ice hockey, men's lacrosse, field
hockey, and amateur boxing.

The American Dental Association estimates that mouthguards prevent
approximately 200,00 injuries each year in high school and collegiate
football. A 1989 study by Mc Nutt et al. found that 75 percent of
oral-facial injuries occurred among athletes who did not wear a mouthguard.
Hard tissue (dental) trauma was found to be 60 times more likely for athletes
who wore no mouthguard than for those who did. In a 1997 study by Kumamoto
et al., athletes learning to slam-dunk a basketball while not wearing a
mouthguard suffered severe dental injuries as a result of entangling their
teeth in the net.

Now, if mouthguards are so great, why don't more children, adolescents, and
college students wear them? The main complaints about mouthguards include:
discomfort, loose fit, difficulty breathing, and difficulty speaking. All of
these problems, however, are found only in over-the-counter mouthguards, which
90 percent of athletes use. A properly fitted mouthguard is protective,
comfortable, resilient, tear resistant, ordorless, tasteless, causes minimal
interference with speaking, has an excellent fit, and is sufficiently thick to
protect the teeth. Only your dentist can provide your child with a properly
fitted mouthguard.

The Four Types Of Mouthguards:

Stock Mouthguards (OTC). Not recommended, due to lack of retention and
lack of protective qualities.

Boil And Bite Mouthguards (OTC). Also not recommended, because they do not
cover and protect all of the back teeth during impact. In addition, they are
too thin to be protective.

Vacuum-Made Mouthguards. These are OK. They are made by the dentist on a
stone cast of the athlete's teeth. A thermoplastic material is adapted to
the stone cast using a special vacuum former. The vacuum-formed mouthguard
is then trimmed and polished.

Pressure Laminated Mouthguards. These are the best! They are custom-made,
multiple-layered mouthguards. High heat and pressure is used to produce a
laminated mouthguard having two or three layers of mouthguard material. This
type of mouthguard will provide a superb fit and great protection for the
athlete.

How To Take Care Of The Mouthguard:

Rinse the mouthguard with cold water or mouth rinse before and after each
use.

Brush and clean the mouthguard periodically with a soft toothbrush and
toothpaste.

Store and protect the mouthguard in a carrying case, and keep the
mouthguard wet during storage.

Avoid contact with hot water, high temperatures, and direct sunlight to
minimize distortion of the mouthguard.

Periodically check the mouthguard for holes or tears, and replace as
needed.

Bring the mouthguard to each dental visit so that your dentist can check
its condition and dental fit.

A recent article in General Dentistry discusses mouthguards:Ranalli DN: Sports dentistry in general practice. General Dentistry.
March-April 2000; 48(2)158-164.